Human/Mouse Sulfamidase/SGSH Alexa Fluor® 647-conjugated Antibody

Catalog #: FAB83801R Datasheet
Catalog # Availability Size / Price Qty
FAB83801R-100UG
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Human/Mouse Sulfamidase/SGSH Alexa Fluor® 647-conjugated Antibody Summary

Species Reactivity
Human, Mouse
Specificity
Detects human Sulfamidase/SGSH in direct ELISAs. Detects human and mouse Sulfamidase/SGSH in Western blots.
Source
Monoclonal Mouse IgG2a Clone # 1018331
Purification
Protein A or G purified
Immunogen
Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Sulfamidase/SGSH
Arg23-Leu502
Accession # P51688
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Label
Alexa Fluor 647 (Excitation= 650 nm, Emission= 668 nm)

Applications

Recommended Concentration
Sample
Western Blot
Optimal dilution of this antibody should be experimentally determined.
 
Immunohistochemistry
Optimal dilution of this antibody should be experimentally determined.
 

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Preparation and Storage

Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

Background: Sulfamidase/SGSH

Also known as N-sulfoglucosamine sulfohydrolase and heparan N-sulfatase, Sulfamidase/SGSH is an important member of the sulfatase family involved in the degradation of heparan sulfate (HS) (1). Different from the HS specific endosulfatases that remove sulfate from internal GlcNAc residues (2), SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. The SGSH deficiency results in mucopolysaccharidosis type IIIA (MPS IIIA, Sanfilippo A syndrome), an autosomal recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations (3). Human SGSH shows 88.6% sequence identity with that of mouse sequence.

Entrez Gene IDs
6448 (Human); 27029 (Mouse)
Alternate Names
heparan sulfate sulfatase; HSS; HSSN-sulphoglucosamine sulphohydrolase; MPS3A; MPS3Asulphamidase; N-sulfoglucosamine sulfohydrolase; SFMD; SFMDEC 3.10.1.1; SGSH; Sulfamidase; Sulfoglucosamine sulfamidase; Sulphamidase

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